title: Expression of myogenic regulatory factors (MRFs) in human neuromuscular disorders.
abstract: Immunohistochemical studies using antibodies to myogenic regulatory factors (MRFs) Myo D, myogenin, myf-5, and myf-6, and transcription factors c-Fos and c-Jun, were performed on muscle biopsies from patients suffering from Duchenne and Becker muscular dystrophies, polymyositis, and denervation atrophy, to investigate whether expression of these factors occurs during degeneration and regeneration of adult muscle fibres. Strong Myo D, myogenin, myf-5 and myf-6 immunoreactivity was observed in the nuclei of small regenerating fibres and satellite cells, as revealed by double-labelling immunohistochemistry with N-CAM antibodies, in Duchenne and Becker muscular dystrophies and in polymyositis. This suggests that the myogenic programme is activated during regeneration of adult human muscle fibres. In addition, strong myf-6 and c-Jun immunoreactivity was found in the cytoplasm of some necrotic muscle fibres in patients with Duchenne and Becker muscular dystrophies and in patients with polymyositis. The latter findings suggest that strong cytoplasmic expression of myf-6 and c-Jun is related to the process of muscle fibre degeneration that occurs in these conditions. Increased Myo D, myogenin, myf-5 and myf-6 immunoreactivity was not observed in the nuclei of denervated muscle fibres, although strong c-Fos and c-Jun immunoreactivity was seen in the nuclei of denervated muscle fibres; this suggests that denervation triggers the expression of these transcription factors. Taken together, these observations demonstrate that MRFs and c-Fos and c-Jun are selectively expressed in different human muscular disorders.
authors.affiliation: Unidad de Patología Neuromuscular, Hospital Príncipes de España, Barcelona, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Olivé
authors.forename: J A
authors.initials: JA
authors.lastname: Martinez-Matos
authors.forename: P
authors.initials: P
authors.lastname: Pirretas
authors.forename: M
authors.initials: M
authors.lastname: Povedano
authors.forename: C
authors.initials: C
authors.lastname: Navarro
authors.forename: I
authors.initials: I
authors.lastname: Ferrer
mesh_terms: D000293:Adolescent; D000328:Adult; D001284:Atrophy / Q000378:metabolism; D002648:Child; D004268:DNA-Binding Proteins; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009121:Muscle Denervation; D009124:Muscle Proteins / Q000378:metabolism; D009136:Muscular Dystrophies / Q000378:metabolism; D017570:MyoD Protein / Q000378:metabolism*; D051787:Myogenic Regulatory Factor 5; D018006:Myogenic Regulatory Factors / Q000378:metabolism*; D018008:Myogenin / Q000378:metabolism*; D009468:Neuromuscular Diseases / Q000378:metabolism*; D017285:Polymyositis / Q000378:metabolism; D016760:Proto-Oncogene Proteins c-fos / Q000378:metabolism; D016755:Proto-Oncogene Proteins c-jun / Q000378:metabolism; D015534:Trans-Activators
chemical_list: D004268:DNA-Binding Proteins; C497417:MYF5 protein, human; C503799:MYOG protein, human; D009124:Muscle Proteins; D017570:MyoD Protein; D051787:Myogenic Regulatory Factor 5; D018006:Myogenic Regulatory Factors; D018008:Myogenin; D016760:Proto-Oncogene Proteins c-fos; D016755:Proto-Oncogene Proteins c-jun; D015534:Trans-Activators; C063403:myogenic factor 6